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fitz 9th > 40 - Eosinophilic Diseases > Flashcards

40 - Eosinophilic Diseases Flashcards

1
Q

Eosinophils develop in the bone marrow from multipotential, stem cell-derived _____+ myeloid progenitor cells

A

CD34

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2
Q

Stimulatory cytokines and growth factors for eosinophils

A

IL-3
GM-CSF
IL-5

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3
Q

Most selective eosinophil-active cytokine, but is relatively late-acting

A

IL-5

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4
Q

Mature eosinophils are slight (smaller/larger) than neutrophils

A

Larger

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5
Q

Five highly basic proteins found within the eosinophil’s cystoplasmic granules

A

Major basic protien-1
Major basic protien-2
Eosinophil-derived neurotoxin/Ribonuclease 2
Eosinophil cationix protien/Ribonuclease 3
Eosinophil peroxidase

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6
Q

Other types of the eosinophil’s cystoplasmic granules

A

Primary granules
Small granules
Secretory vesicles

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7
Q

Characteristically found in asthmatic sputum and in feces from patients with helminth infections or eosinophilic gastroenteritis

A

Charcot-Leyden crystal protein or galectin-10

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8
Q

Charcot-Leyden crystal protein can also be found in

A

Neutrophils

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9
Q

Charcot-Leyden crystal protein is contained in

A

Primary granules

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10
Q

More common in immature eosinophilic promyelocyes

A

Primary granules

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11
Q

Contain acid phosphatase and arylsulfatase

A

Small granules

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12
Q

Also referred to as tubulovesicular structures or microgranules, are characterized by their small, dumbbell-shaped appearance and their albumin content

A

Secretory vesicles

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13
Q

Most abundant granules in number

A

Secretory vesicles

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14
Q

Principal stores of arachidonic acid; aso contain the enzymes cyclooxygenase, 5- and 15-lipoxygenase

A

Lipid bodies

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15
Q

Y/N: Eosinophils are present in blood permanently.

A

No - transiently, ranging from 8-18 hours

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16
Q

Eosinophils comprise _____% or less of circulating leukocytes

A

6

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17
Q

Eosinophils are primarily tissue-dwelling cells, with an average life span of

A

2-5 days; may reach up to 14 days

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18
Q

Only tissues in which eosinophils normally reside

A 
Bone marrow
Spleen
Lymph node
Thymus
Gastrointestinal tract from the stomach to the colon, sparing the esophagus
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19
Q

Only organ other than the bine marrow in which extracellular eosinophil granule protein deposition is observed even under homeostatic conditions

A

Gastrointestinal tract

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20
Q

Eosinophils and their granule proteins are found in the _____ in normal gastrointestinal tract and are not found in _____

A

Lamina propria

Peyer patches or epithelium

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21
Q

The recruitment of eosinophils to the gastrointestinal, thymic, uterine, and mammary tissues is under the control of

A

CCL11

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22
Q

Eosinophils can migrate to areas of tissue injury or necrosis through the _____ released from necrotic cells

A

High-mobility group box-1 protein (HGMB1)

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23
Q

Y/N: Once eosinophils enter tissues, most do not recirculate

A

Yes

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24
Q

Among the products of eosinophils that are most damaging to the host are the

A

Cationic proteins (EPO, ECO, EDN, MBP-1)

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25
Q

All 4 of the cationic proteins (EPO, ECP, EDN, MBP-1) likely contribute to the edema observed in skin diseases because of their

A

Vasodilatory effect

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26
Q

Eosiniphil granule proteins induces direct tissue damage to

A

Host cellls and microbes

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27
Q

Comprises the crystalloid core of the specific eosinophil granule

A

Major basic protein

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28
Q

_____, but none of the other eosinophil granule proteins, stimulate histamine and LTC4 release from human basophils

A

MBP-1 and MBP-2

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29
Q

Key participant in generating reactive oxidants and free radical species in activated eosinophils

A

Eosinophil peroxidase

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30
Q

Protein that is present in the highest molar concentration in eosinophil granules

A

MBP

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31
Q

By weight, is the most abundant protein constituting approximatelt 25% of the specific eosinophil granule’s total protein mass

A

EPO

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32
Q

Play a role in viral host defense to RNA viruses

A

ECP and EDN

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33
Q

Functions as an alarmin, alerting the adaptive immune system to preferentially enhance antigen-specific Th2 responses

A

EDN

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34
Q

Y/N: No single surface protein is uniquely expressed in eosinophils

A

Yes

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35
Q

Most important selectin pair in eosinophil migration into tissues

A

P-selectin and PSGL-1

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36
Q

Among the nonselective drugs for eosinophil reduction, _____ generally are very effective

A

Glucocorticoids

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37
Q

Y/N: Glucocorticoids cause an immediate reduction in circulating eosinophils.

A

Yes - within 3 hours

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38
Q

Mutation in myeloproliferative hypereosinophilic syndrome (chronic eosinophilic leukemia)

A

FIP1L1-PDGFRA

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39
Q

Approved for the treatment of chronic myelogenous leukemia and hypereosinophilic syndrome

A

Imatinib mesylate

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40
Q

Monoclonal antibody to CD52; used in the treatment of chronic (B-cell) lymphocytic leukemia and T-cell lymphoma

A

Alemtuzumab

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41
Q

Eosinophils, but not _____, also express CD52

A

Neutrophils

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42
Q

First humanized monoclonal antibody against IL-5

A

Mepolizumab

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43
Q

Anti-IL-5 receptor alpha humanized monoclonal antibody

A

Benralizumab

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44
Q

Y/N: Pathogenic influence of eosinophils may be unrelated to their numbers in tissues

A

Yes

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45
Q

Revised diagnostic criteria for hypereosinophilic syndromes: blood eosinophilia > _____ eosinophils/mm3 on at least 2 separate determinations or evidence of prominent tissue eosinophilia associated with symptoms and marked blood eosinophilia

A

1500

46
Q

Patients with myeloproliferative hypereosinophilic syndrome frequently present with clinical features resembling those of

A

Chronic myelogenous leukemia

47
Q

Although chromosomal abnormalities characterize this subtype of HES, the disease may evolve into definite leukemia

A

Myeloproliferative HES

48
Q

Patients with _____ HES abnormal T-cell clones with unusual surface phenotypes

A

Lymphocytic

49
Q

An 800-kilobase deletion on chromosome band 4q12 that codes for a tyrosine kinase has been found in _____ HES

A

Myeloproliferative

50
Q

The FIP1L1-PDGFRA gene is detected in

A

Mast cells
Eosinophils
Neutrophils
Mononuclear cells

51
Q

Myeloproliferative HES with documented mutations is known as

A

Chronic eosinophilic leukemia

52
Q

Increased serum levels of IgE are often present in _____ HES

A

Lymphocytic

53
Q

Levels of vitamin B12 and tryptase may be increased in _____ HES

A

Myeloproliferative

54
Q

Other than in _____, vasculitis only rarely is associated with HES

A

Eosinophilic granulomatosis with polyangitis

55
Q

Along with eosinophilia, _____ commonly are found in helminthic infections

A

Total serum IgE levels higher than 500 IU/mL

56
Q

In patients with isolated urticarial plaques with or without angioedema, the differential diagnosis includes common and persistent urticaria, but demonstration of _____ supports HES

A

Multiorgan involvement

57
Q

HES with episodic angioedema may resemble hereditary angioedema clinically, although patients with hereditary angioedema have a family history of the disease and rarely have the _____ that characterize HES

A

Markedly elevated eosinophil counts

58
Q

Major cause of death in HES

A

Congestive heart failure from restrictive cardiomyopathy

Followed by sepsis

59
Q

Corticostetoids are considered the first-line therapy in HES patients without the gene mutation, once _____ has been excluded

A

Strongyloides infection

60
Q

Myeloproliferative HES is responsive to

A

Imatinib

61
Q

Adverse effect of imatinib

A

Endomyocardial disease may worsen during the first several days of treatment

62
Q

Should be monitored before and during imatinib therapy

A

Tropinin levels

63
Q

To improve cardiac function, _____ should be given before and with initiation of imatinib therapy

A

Glucocorticoids

64
Q

Individual lesions gradually change from bright red to brown-red and then blue-gray or greenish-gray, resembling morphea

A

Wells syndrome

65
Q

Most frequent systemic complain in Wells syndrome

A

Malaise

66
Q

A nonspecific hypersensitivity reaction in response to exogenous and/or endogenous stimuli is regarded as the most important pathomechanism in

A

Wells syndrome

67
Q

Characterized by diffuse dermal infiltration with eosinophils, histiocytes, and foci of amorphous and/or granular material associated with connective tissue fibers

A

Wells syndrome

68
Q

Y/N: Flame figured is diagnostic of Wells syndrome

A

No - characteristic for, but not diagnostic of

69
Q

Hallmark of Wells syndrome

A

Flame figures

70
Q

Prognosis of Wells syndrome

A

Resolves without scarring, usually within weeks to months, but multiple recurrences are common

71
Q

Angiolymphoid hyperplasia with eosinophilia shows a predilection for the

A

Head and neck area, including the ears

72
Q

ALHE has been reported to occur in _____, which implies thst sex hormones may be a factor in its development

A

Pregnancy

73
Q

Also has developed in patients with T-cell clonality, which suggets that it may be an early or low-grade T-cell lymphoma

A

ALHE

74
Q

Well-defined area in the dermis and/or subcutis of prominent vascular proliferation with large epithelioid or histiocytoid endothelial cells that contain abundant eosinophilic cytoplasm, often with cytoplasmic vacuoles

A

ALHE

75
Q

The stroma of ALHE is typically

A

Myxoid

76
Q

KD vs ALHE: lesions

A

ALHE - smaller, more superficial, more numerous, often asymptomatic

77
Q

Peripheral blood eosinophilia and increase IgE levels in (KD/ALHE)

A

KD

78
Q

Dominant histologic feature is lymphoid proliferation, often with germinal centers with prominent infiltration of eosinophils. Fibrosis is characteristic, and eosinophil abscesses may occur

A

KD

79
Q

Represent the dominant characteristic of KD

A

Lymphoid follicles

80
Q

KD vs ALHE: nephropathy

A

KD - present in up to 20% of patients

82
Q

ALHE course

A

Chronic and nonremitting

83
Q

KD treatment of choice

A

Surgical excision

84
Q

Variants of eosinophilic pustular folliculitis

A
  1. Classical EPF
  2. Immunosuppresion-associated EPF
  3. Infantile EPF
85
Q

Immunosuppression EPF may be subdivided into

A
  1. HIV-associated type

2. Malignancy-associated type

86
Q

Presents as recurrent crops or clusters of follicular papules and pustules, which form an annular pattern and usually resolve in 7-10 days; predominantly on the face and trunk

A

Classical EPF

87
Q

Infantile type of EPF typically are located on the

A

Scalp

88
Q

Extremely pruritic discrete follicular papules, typically involving thr head and neck and often the proximal extremities

A

HIV-associated EPF

89
Q

Although patients with classical EPF usually have eosinophilia with leukocytosis, HIV-positive patients often exhibit

A

Eosinophilia with lymphopenia

90
Q

Elevated serum IgE is uncommon in _____ EPF

A

Infantile

91
Q

Infiltration of eosinophils into hair follicles and perifollicular areas, sometimes with follicular damage

A

EPF

92
Q

EPF prognosis based on type

A

Infantile - good

Classical and HIV-associated - recurrences

93
Q

First approach to the treatment of all types of EPF

A

Topical glucocorticoids
Topical calcineurin inhibitors
Also NSAIDs, particularly indomethacin

94
Q

Papuloerythroderma of Ofuji frequently occur in

A

Elderly males, especially in the eighth or ninth decade

95
Q

Itchy erythroderma-like eruptions formed by the coalescence of flat-topped, red-to-brown papules with a cobblestone appearance

A

Papuloerythroderma of Ofuji

96
Q

Papuloerythroderma of Ofuji affects the

A

Limbs and trunk

97
Q

Sparing of the abdominal skin folds

A

Deck-chair sign

98
Q

Y/N: Papuloerythroderma of Ofuji affects the mucous membranes, hair, and nails

A

No - Mucous membranes, hair, and nails are always spared

99
Q

Approximately 20% of cases of papuloerythroderma of Ofuji are associated with

A

Hematologic neoplasms or

Visceral malignancies

100
Q

Deck-chair sign is observed in

A

Papuloerythroderma of Ofuji

Other erythrodermas, such as psoriasis and atopic dermatitis

101
Q

Histopathology of papuloerythroderma of Ofuji

A

Nonspecific spongiotic dermatitis-like patterns

102
Q

Papuloerythroderma of Ofuji course

A

Chronic and non-self-limiting

103
Q

Y/N: Papuloerythroderma of Ofuji is frequently refractory to treatment

A

Yes

104
Q

Asymptomatic red, brown, or violaceous plaques that are soft, smooth, and well circumscribed, often showing follicular s accentuation and telangiectasia

A

Granuloma faciale

105
Q

Can be considered a localized chronic fibrosing vasculitis

A

Granuloma faciale

106
Q

Normal-appearing epidermis, narrow grenz zone, dense and diffuse infiltrate of lymphocytes, plasma cells, eosinophils, and neutrophils with evidence of leukocytoclasis

A

Granuloma faciale

107
Q

Primary histologic differential diagnosis of granuloma faciale

A

Erythema elevatum diutinum

108
Q

Granuloma faciale vs EED: histology

A

Grenz zone is not typical of EED
Eosinophils and plasma cells are more prominent in granuloma faciale
Neutrophils are more frequently found in EED

109
Q

Granuloma faciale course

A

Chronic and rarely resolves spontaneously

110
Q

Depression along the course of the superficial vein that is more marked upon the elevation of the affected limb

A

Groove sign

111
Q

Groove sign is a characteristic finding of

A

Eosinophilic fasciitis

112
Q

Historically related to ingestion of certain lots of L-tryptophan

A

Eosinophilia-myalgia syndrome

113
Q

Dermoscopic finding of ALHE

A

Polymorphous vascular pattern

fitz 9th (108 decks)

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